colin farquharson - what becomes of the broken hearted?

Takotsubo Cardiomyopathy

COLIN FARQUHARSON

Patient 72 year old ladyExtensive PMHPrevious DU 1972 / 2002COPDPernicious AnaemiaCKD stage IIICrohns Disease previous right Hemicolectomy, underwent laparotomy and excision of perforated recurrent ileal / ileocolic Crohns plus loop ileostomy 31st August 2008

Admitted under Medicine with nausea / vomiting / high stoma output 17 Oct 2008Severe hyponatraemia (117mmol/L), Mg also low at 0.27, CRP 25, plts 758, Stool giardial cysts!

Initial diagnosis Dehydration with electrolyte imbalance secondary to nausea and vomitingIncidental giardiasisCrohns flare-up

Treated with IV fluids / oral magnesium / oral metronidazole initial good improvement

Transferred under Gastroenterologists

23 Oct 2009 deteriorated with worsening diarrhoea / signs of LRTI

Pyrexial, Sinus tachycardia 120bpm, BP 120/64mmHg

CRP 137, WCC 30.2, Mg 0.30, Ca 1.38

CXR Left pneumonic consolidationAXR not diagnostic

Rx PO metronidazole / IV Tazocin / Mg & Ca replacement

CT Abdo no worrying intra-abdominal pathology

Made slow but steady recovery over next few weeks

Due to be commenced on IV TPN 11 Nov 2009 to improve nutritional status

Had some pain / distress during central line insertion 11 Nov 2009, then developed sudden onset malaise / feeling of impending doom

Transient pre-syncopal episode only lasted 1-2 mins

HR 108bpm>140bpm SR, BP 142/62mmHg

No clinical signs of heart failure

Hb 8.0 g/dl, Cr 100, Mg 0.67, Cr 2.12

ECG

ECG Baseline on admission

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ECG 11/11/2008 21:35

ECG 11/11/2008 22:05

ECG 12/11/2008 09:30

ECG 13/11/2008 10:30

Treated initially as Acute STEMI 11 Nov 2008

Not thrombolysed as recent major surgery / absence of chest pain

Referred for bail-out PCI at Hull was not accepted due to comorbidities (main issue being significant anaemia)

Rx Aspirin / Clopidogrel / IV Nitrates / blood transfusion / IV fluids

Troponin I elevated at 12 hours at 1.06, then dropped to 0.13 within 24 hours

CK not elevated at any stage 60 maximally

No pain, but worsening dyspnoea / hypotension despite above therapy

Coronary angiogram organised for 14 Nov 2008

CORONARY ANGIOGRAMShowed angiographically normal wide calibre coronary arteries with no significant flow-limitation in any arterial segment

Left ventriculography showed substantial LV apical ballooning with overall mild-moderate LV impairment and elevated LVEDP

Diagnosis TAKOTSUBO CARDIOMYOPATHYLikely triggered by dramatically elevated catecholamine levels caused by concurrent illness in a post-menopausal woman

Rx entirely supportive

Commenced on beta-blocker / ACEI, continued on Aspirin / SC prophylactic clexane

IV TPN continued

Continued to recover with GI supportive therapy

No further cardiac complications

Discharged home 08 Dec 2008

ECHO end Dec 2008 Normal LV systolic function with resolution of apical ballooning

PROGRESSNo subsequent GI complications stoma stable with no further vomiting / stoma problems

No further cardiac complications no recurrence of chest pain / cardiac failure

However, patient readmitted with exacerbation of COPD secondary to pseudomonal pneumonia 08 Jan 2009 developed Type II respiratory failure and died in ITU 16 Jan 2009

Takotsubo CardiomyopathyColin A J FarquharsonConsultant CardiologistDiana Princess of Wales HospitalGrimsbyUnited Kingdom

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Takotsubo CardiomyopathyCardiomyopathy characterized by transient apical and midventricular LV dysfunction in the absence of significant coronary artery disease that is triggered by emotional or physical stress.In setting of depressed/abnormal function of distal and apical LV segments there is compensatory hyperkinesis of basal walls ballooning of apex during systole.Typically recover normal LV function in 1-4 weeks.

Colin Farquharson , Cardiologist , Grimsby UK

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Takotsubo Cardiomyopathy1st described in Japan in 1990Named after the tako-tsubo, which is an octopus trapShape of the trap is similar to the appearance of LV apical ballooning noted in patients with this form of cardiomyopathy

Was later described in many other reports and was subsequently recognised as a distinct entity


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Takotsubo Cardiomyopathy

Kurisu, S., et al. 2002. American Heart Journal. 143: 448-455.Colin Farquharson , Cardiologist , Grimsby UK

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DIFFERENCES IN LV MORPHOLOGY

NORMAL LEFT VENTRICULAR CONTRACTIONABNORMAL CONTRACTION OF LV TAKING THE SHAPE OF A TAKO-TSUBOColin Farquharson , Cardiologist , Grimsby UK

AliasesTakotsubo cardiomyopathyStress-induced cardiomyopathyTransient left ventricular apical ballooning syndrome Apical ballooning syndromeBroken heart syndromeAmpulla cardiomyopathyColin Farquharson , Cardiologist , Grimsby UK

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Takotsubo CardiomyopathyMay account for up to 2% of suspected ACSIn-hospital mortality ranges between 0-8%Much more common in women (~90%), especially postmenopausal women (>80% of cases)Mean age 58-75 yearsMore common in industrialised nationsMany recognised triggers: death of loved one, other catastrophic news, devastating financial losses, natural disasters, physical illness/ICU, etc.Colin Farquharson , Cardiologist , Grimsby UK

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Proposed Diagnostic CriteriaTransient a/dyskinesis of apical and midventricular segments in association with regional wall motion abnormalities that extend beyond the distribution of a single epicardial vesselAbsence on angiography of obstructive coronary artery disease or evidence of acute plaque ruptureNew ST segment elevation or T wave inversions on ECGAbsence of recent significant head trauma, intracranial bleeding, phaeochromocytoma, myocarditis, or hypertrophic cardiomyopathyProposed by Bybee, et al. 2004. Annals of Internal Medicine. 141: 858-865.Colin Farquharson , Cardiologist , Grimsby UK

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Recognised emotional stress triggersEmotional stressDeath or severe illness or injury of family member, friend, petReceiving bad news diagnosis of major illness, family divorce, spouse leaving for warSevere argumentFear of public speakingInvolvement of legal proceedingsFinancial loss business / gamblingCar accidentSurprise partyMoving to new houseColin Farquharson , Cardiologist , Grimsby UK

Recognised physical stress triggersNon-cardiac surgery / procedure e.g. cholecystectomy / hysterectomySevere illness asthma / COPD, connective tissue disorders, inflammatory bowel diseaseIllnesses that cause pain fracture, renal colic, pneumothorax, PERecovery from general anaesthesiaCocaine abuseOpiate withdrawalStress testing dobutamine stress echo / MPSThyrotoxicosisColin Farquharson , Cardiologist , Grimsby UK

New England Journal of Medicine 2005;352:539-548Colin Farquharson , Cardiologist , Grimsby UK

New England Journal of Medicine 2005;352:539-548


(Postulated) PathogenesisCatecholamine excess Norepinephrine levels are very elevated in ~75% in some studiesPlasma catecholamines significantly higher than in cases of MIMay induce microvascular spasm or dysfunction myocardial stunning or direct myocardial toxicityLimited endomyocardial biopsy data c/w histologic signs of catecholamine toxicityCoronary artery spasm or microvascular spasmMyocarditisPost-menopausal downregulation of oestrogen receptorsDense distribution of cardiac adrenoceptors in LV apex of women > menColin Farquharson , Cardiologist , Grimsby UK

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Presentation (similar to acute MI)Substernal chest pain (but not always)ECG abnormalitiesST elevation (usually anterior leads)- 82%ST depressionT wave inversionQT prolongationAbnormal Q wavesElevated cardiac biomarkersDyspnoeaShock similar haemodynamically to cardiogenic shockSyncope / feeling of doomColin Farquharson , Cardiologist , Grimsby UK

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CARDIAC TROPONIN RELEASE ABS VS MI


Acute ComplicationsTachyarrhythmias, bradyarrhythmiasPulmonary oedemaCardiogenic shockTransient LV outflow tract obstructionMitral valve dysfunctionAcute thrombus formation and strokeDeath


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EvaluationBecause presentation is similar to ACS, management is usually similar in initial stages.LV ventriculogram and/or echocardiography can both be used to visualize apical ballooning with a/dyskinesis of apical to of the LV.Average LV EF range 20-49%Can have atypical ballooning of the middle or basal portions of the LV (much less common)Wall motion abnormalities typically involve the distribution of more than one coronary arteryVentriculography and echocardiography also allow evaluation for LV outflow tract obstruction (~16%).Cardiac catheterization reveals lack of flow limiting coronary lesions or evidence of plaque rupture.Colin Farquharson , Cardiologist , Grimsby UK

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ManagementSupportive, conservative therapyHydrate, remove / reduce stress (if possible)Treat LV dysfunction with standard heart failure regimen- including beta blocker, ACE inhibitor, diuretics (if volume overloaded), aspirinUsually treated for ~6 monthsFor pts who are hypotensive with shock, perform echo to evaluate for LVOT obstruction.No LVOT obstruction inotropes, IABP if needed+LVOT obstruction NO inotropes (can worsen obstruction), use beta blockers (+/- -agonist phenylephrine), IABP if needed+/- fluid resuscitation (evaluate pulmonary status)Colin Farquharson , Cardiologist , Grimsby UK

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Prognosis0-8% in-hospital mortality, likely closer to 1-2% if optimally treatedRecovery of LV function, typically in 1-4 weeksLate sudden death (rare) and recurrent disease (

colin farquharson - what becomes of the broken hearted?

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